Article

Pediatric Cardiology

, Volume 29, Issue 2, pp 309-312

Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease

  • O. C. OnyekwereAffiliated withCenter for Sickle Cell Disease, Howard University Email author 
  • , A. CampbellAffiliated withCenter for Sickle Cell Disease, University of Michigan
  • , M. TeshomeAffiliated withCenter for Sickle Cell Disease, University of Michigan
  • , S. OnyeagoroAffiliated withCenter for Sickle Cell Disease, Howard University
  • , C. SylvanAffiliated withCenter for Sickle Cell Disease, Howard University
  • , A. AkintiloAffiliated withCenter for Sickle Cell Disease, Howard University
  • , S. HutchinsonAffiliated withCenter for Sickle Cell Disease, University of Michigan
  • , G. EnsingAffiliated withCenter for Sickle Cell Disease, University of Michigan
  • , P. GaskinAffiliated withDepartment of Pediatrics and Child Health, Howard UniversityPediatric Cardiology, University of Maryland
    • , G. KatoAffiliated withVascular Therapeutics Section, National Heart, Lung and Blood Institute, and the Critical Care Medicine Department, Clinical Center, National Institutes of Health
    • , S. RanaAffiliated withDepartment of Pediatrics and Child Health, Howard University
    • , J. KwagyanAffiliated withGeneral Clinical Research Center, Howard University
    • , V. GordeukAffiliated withCenter for Sickle Cell Disease, Howard UniversityGeneral Clinical Research Center, Howard University
    • , J. WilliamsAffiliated withCenter for Sickle Cell Disease, University of Michigan
    • , O. CastroAffiliated withCenter for Sickle Cell Disease, Howard University

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Abstract

The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV ≥2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV ≥3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD.

Keywords

Hemolysis Pulmonary disease Sickle cell disease Tricuspid valve regurgitation